Scleroderma patients with pulmonary problems face higher mortality risk

A new study strongly links pulmonary complications to survival rates among scleroderma patients.

Results of the study, published online in Arthritis & Rheumatology, show that patients who developed pulmonary hypertension within three years of being diagnosed with scleroderma had a mortality risk nearly seven times higher than patients without pulmonary hypertension. Pulmonary fibrosis more than doubled the risk of death among scleroderma patients.

The study focused on 398 scleroderma patients at the Center for Rheumatology and Connective Tissue Diseases at Royal Free Hospital in London from 1995 to 1999, with a 15-year follow-up. Two hundred fifty-two of the patients studied had the limited cutaneous form of scleroderma, while the remaining 146 had the diffuse cutaneous form.

Multivariate analysis showed that certain factors could predict mortality, including advanced age at disease onset, diffuse disease, low carbon monoxide diffusion capacity, low hemoglobin, elevated creatinine, and the presence of cardiac involvement or pulmonary hypertension.

Following the analysis, the authors created a system for scoring risk factors in patients. Factors such as age of disease onset, type of scleroderma and others were assigned a point value. Mortality risk was then assessed and analyzed based on score.

The results of the study contradict some long-held assumptions. Previously, pulmonary hypertension was generally considered a late development in systemic sclerosis, but in the new study, cases were noticed in the first three years. Another contradiction is that pulmonary hypertension was believed to be much more strongly associated with limited, rather than diffuse, scleroderma, but the incidences were similar in the recent study.

“The predictive models we present could be used as clinical tools for patient risk stratification and could facilitate cohort enrichment for event driven studies,” the authors wrote.